Vanishing bile duct syndrome
Vanishing bile duct syndrome (or "ductopenia") is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia.[1]
Etiology
Congenital/Developmental
In fetal and neonatal life the ductal plates are remodeled. The malformations can be atretic or fibrocystic.
Atretic causes
- Intrahepatic bile duct atresia (Alagille syndrome) (ALGS2 MIM:610205 and ALGS1 MIM:118450)
- Extrahepatic bile duct atresia
Fibrocystic causes
- Autosomal recessive polycystic kidney disease
- Congential hepatic fibrosis
- Caroli's disease
- Von Meyenburg complex
Chromosomal associations
- Trisomy 17, 18 and 21
Genetic associations
- Cystic fibrosis
- Alpha 1 antitrypsin deficiency
- Trihydroxycoprostanic acidemia
- Byler's disease
Immunologic associations
Bile duct injury and loss can result from autoimmune destruction. T cells recognize biliary epithelial cell antigens causing injury and eventual atresia.
Other causes
- Primary biliary cirrhosis
- Primary sclerosing cholangitis
- Hodgkin's lymphoma [2]
- Chronic graft-versus-host disease
- Drugs(chlorpromazine)/Toxins
- Ischemia
Clinical presentation
The presentation is dependent upon the underlying etiology. The course can be rapid or chronic.
Symptoms
- Fatigue
- Anorexia
- Abdominal pain
- Weight loss
- Pruritus
Signs
- Hyperlipidemia
- Malabsorption
- Fat-soluble vitamin deficiencies
- Elevated alkaline phosphatase
- Elevated gamma-glutamyltransferase
- Elevated conjugated bilirubin
Treatment
Treatment is dependent upon the underlying etiology. Treatment is supportive as it is not possible to induce regrowth of lost ducts.
Medical therapies
- Ursodeoxycholic acid
- Immunosuppression
- General consensus is that more studies are needed before this can be considered
- Organ transplant
References
- ↑ Reau NS, Jensen DM (February 2008). "Vanishing bile duct syndrome". Clin Liver Dis. 12 (1): 203–17, x. doi:10.1016/j.cld.2007.11.007. PMID 18242505.
- ↑ Rossini, M. S.; Lorand-Metze, I; Oliveira, G. B.; Souza, C. A. (2000). "Vanishing bile duct syndrome in Hodgkin's disease: Case report". Sao Paulo medical [Revista paulista de medicina]. 118 (5): 154–7. PMID 11018850.
External links
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